The DOI matches its Crossref record; 23 of the 23 references it cites were found in a registry or an index.
Restoring Cystic Fibrosis Transmembrane Conductance Regulator Function Reduces Airway Bacteria and Inflammation in People with Cystic Fibrosis and Chronic Lung Infections
Open accessAbstract
RATIONALE: Previous work indicates that ivacaftor improves cystic fibrosis transmembrane conductance regulator (CFTR) activity and lung function in people with cystic fibrosis and G551D-CFTR mutations but does not reduce density of bacteria or markers of inflammation in the airway. These findings raise the possibility that infection and inflammation may progress independently of CFTR activity once cystic fibrosis lung disease is established. OBJECTIVES: To better understand the relationship between CFTR activity, airway microbiology and inflammation, and lung function in subjects with cystic fibrosis and chronic airway infections. METHODS: We studied 12 subjects with G551D-CFTR mutations and chronic airway infections before and after ivacaftor. We measured lung function, sputum bacterial content, and inflammation, and obtained chest computed tomography scans. MEASUREMENTS AND MAIN RESULTS: Ivacaftor produced rapid decreases in sputum Pseudomonas aeruginosa density that began within 48 hours and continued in the first year of treatment. However, no subject eradicated their infecting P. aeruginosa strain, and after the first year P. aeruginosa densities rebounded. Sputum total bacterial concentrations also decreased, but less than P. aeruginosa. Sputum inflammatory measures decreased significantly in the first week of treatment and continued to decline over 2 years. Computed tomography scans obtained before and 1 year after ivacaftor treatment revealed that ivacaftor decreased airway mucous plugging. CONCLUSIONS: Ivacaftor caused marked reductions in sputum P. aeruginosa density and airway inflammation and produced modest improvements in radiographic lung disease in subjects with G551D-CFTR mutations. However, P. aeruginosa airway infection persisted. Thus, measures that control infection may be required to realize the full benefits of CFTR-targeting treatments.
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Cites
Random-Effects Models for Longitudinal Data
Laird, N. M., & Ware, J. H. (1982) · Biometrics · doi:10.2307/2529876 · Cited by 8,923
Update on Key Emerging Challenges in Cystic Fibrosis
Jennings, M. T., Riekert, K. A., & Boyle, M. P. (2014) · Medical Principles and Practice · doi:10.1159/000357646 · Cited by 3,648
BIGSdb: Scalable analysis of bacterial genome variation at the population level
Jolley, K. A., & Maiden, M. C. (2010) · BMC Bioinformatics · doi:10.1186/1471-2105-11-595 · Cited by 2,368
A CFTR Potentiator in Patients with Cystic Fibrosis and the G551D Mutation
Ramsey, B. W., Davies, J., McElvaney, N. G., Tullis, E., Bell, S. C., Dřevínek, P., Griese, M., McKone, E. F., Wainwright, C. E., Konstan, M. W., et al. (2011) · New England Journal of Medicine · doi:10.1056/nejmoa1105185 · Cited by 2,195
Self-citationPathophysiology and Management of Pulmonary Infections in Cystic Fibrosis
Gibson, R. L., Burns, J. L., & Ramsey, B. W. (2003) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.200304-505so · Cited by 1,633
Intermittent Administration of Inhaled Tobramycin in Patients with Cystic Fibrosis
Ramsey, B. W., Pepe, M. S., Quan, J. M., Otto, K. L., Montgomery, A. B., Williams-Warren, J., Vasiljev-K, M., Borowitz, D., Bowman, C. M., Marshall, B. C., et al. (1999) · New England Journal of Medicine · doi:10.1056/nejm199901073400104 · Cited by 1,300
Cystic Fibrosis Pulmonary Guidelines: Chronic Medications for Maintenance of Lung Health
Flume, P. A., O'Sullivan, B. P., Robinson, K. A., Goss, C. H., Mogayzel, P. J., Willey-Courand, D. B., Bujan, J., Finder, J., Lester, M., Quittell, L., et al. (2007) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.200705-664oc · Cited by 1,024
Effect of VX-770 in Persons with Cystic Fibrosis and the G551D- CFTR Mutation
Accurso, F. J., Rowe, S. M., Clancy, J. P., Boyle, M. P., Dunitz, J. M., Durie, P. R., Sagel, S. D., Hornick, D. B., Konstan, M. W., Donaldson, S. H., et al. (2010) · New England Journal of Medicine · doi:10.1056/nejmoa0909825 · Cited by 807
Self-citationCystic Fibrosis Pulmonary Guidelines: Chronic Medications for Maintenance of Lung Health
Mogayzel, P. J., Naureckas, E. T., Robinson, K. A., Mueller, G., Hadjiliadis, D., Hoag, J. B., Lubsch, L., Hazle, L., Sabadosa, K., Marshall, B., et al. (2013) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201207-1160oe · Cited by 615
Rowe, S. M., Heltshe, S. L., Gonska, T., Donaldson, S. H., Borowitz, D., Gelfond, D., Sagel, S. D., Khan, U., Mayer-Hamblett, N., Van Dalfsen, J. M., et al. (2014) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201404-0703oc · Cited by 492
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Cited by
Shteinberg, M., Haq, I. J., Polineni, D., & Davies, J. C. (2021) · The Lancet · doi:10.1016/s0140-6736(20)32542-3 · Cited by 682
The dynamic lung microbiome in health and disease
Natalini, J. G., Singh, S., & Segal, L. N. (2023) · Nature Reviews Microbiology · doi:10.1038/s41579-022-00821-x · Cited by 523
Nick, J. A., Dedrick, R. M., Gray, A. L., Vladar, E. K., Smith, B. E., Freeman, K. G., Malcolm, K. C., Epperson, L. E., Hasan, N. A., Hendrix, J., et al. (2022) · Cell · doi:10.1016/j.cell.2022.04.024 · Cited by 251
Self-citationThe impact of CFTR modulator therapies on CF airway microbiology
Rogers, G. B., Taylor, S. L., Hoffman, L. R., & Burr, L. D. (2020) · Journal of Cystic Fibrosis · doi:10.1016/j.jcf.2019.07.008 · Cited by 52
Self-citationRapid therapeutic advances in CFTR modulator science
Clancy, J. P. (2018) · Pediatric Pulmonology · doi:10.1002/ppul.24157 · Cited by 52
Metadata
- Authors
- Hisert, K. B., Heltshe, S. L., Pope, C., Jorth, P., Wu, X., Edwards, R. M., Radey, M., Accurso, F. J., Wolter, D. J., Cooke, G., Adam, R. J., Carter, S., Grogan, B., Launspach, J. L., Donnelly, S. C., Gallagher, C. G., Bruce, J. E., Stoltz, D. A., Welsh, M. J., Hoffman, L. R., McKone, E. F., & Singh, P. K.
- Venue
- American Journal of Respiratory and Critical Care Medicine
- Publisher
- Oxford University Press (OUP)
- Published
- 2017-06-15
- Volume
- 195
- Issue
- 12
- Pages
- 1617-1628
- ISSN
- 1073-449X (ISSN-L)
- 1535-4970
- OpenAlex
- W2589766919
- Cited by
- 389
- References
- 23
- FWCI
- 24.70
- Type
- journal article
- Language
- en
- Open access
- green
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- Hisert, K. B., Heltshe, S. L., Pope, C., Jorth, P., Wu, X., Edwards, R. M., Radey, M., Accurso, F. J., Wolter, D. J., Cooke, G., Adam, R. J., Carter, S., Grogan, B., Launspach, J. L., Donnelly, S. C., Gallagher, C. G., Bruce, J. E., Stoltz, D. A., Welsh, M. J., . . . Singh, P. K. (2017). Restoring Cystic Fibrosis Transmembrane Conductance Regulator Function Reduces Airway Bacteria and Inflammation in People with Cystic Fibrosis and Chronic Lung Infections. American Journal of Respiratory and Critical Care Medicine. https://doi.org/10.1164/rccm.201609-1954oc
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Bibliographic data: Semantic Scholar, OpenAlex, Crossref.
Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.
